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Chromophobe renal cell carcinoma (CRCC) is a rare variant of renal carcinomas arising from the intercalated cells of the distal renal tubule and representing 5% among all renal tumors. Its biological behaviour is variable, less aggresive than clear cell renal carcinoma. Histochemical, ultrastructural and molecular genetic characteristics are different from other renal carcinomas. Age at presentation is about the 6th decade of life. We report an exceptional 10 year-old boy case with a CRCC. Diagnostic and therapeutic aspects for the management of this tumor are reviewed.

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